Background: Quality of life and long-term prognosis are frequently, often severely impaired in craniopharyngioma (CP) patients.
Objective and hypotheses: Knowledge of risk factors for long-term outcome is important for optimisation of treatment.
Method: Overall survival (OS) and progression-free survival (PFS), BMI, neuropsychological status (EORTCQLQ-C30, MFI-20), and psychosocial status were analysed in 261 patients with childhood-onset CP diagnosed before 2000 and longitudinally observed in HIT-Endo.
Results: 20-years OS was lower (P=0.006) in CP with hypothalamic involvement (HI) (n=132, 0.84±0.04) when compared to CP without HI (n=82, 0.95±0.04). OS was not related to degree of resection, gender, or diagnosis age or year (before/after 1990). PFS (n=168, 0.58±0.05) was lower in younger (age <5 years at diagnosis) (n=30, 0.39±0.10) compared with patients 510 years (n=66, 0.52±0.08) and >10 years (n=72, 0.77±0.06). PFS was not associated with HI, degree of resection, nor gender. HI led to severe weight gain during the first 812 years of follow-up (median BMI increase: +4.59 S.D.) compared to no HI (median increase: +1.20 S.D.) (P=0.00). During >12 years follow-up, patients with HI presented no further increase in BMI. QoL in CP patients with HI was impaired by obesity, physical fatigue, reduced motivation, dyspnoea, diarrhoea, and non-optimal psychosocial development.
Conclusion: OS and QoL are impaired by HI in long-term survivors of CP. HI is associated with severe obesity, plateauing after 12 years. OS/PFS are not related to degree of resection, but gross-total resection should be avoided in cases of HI to prevent further hypothalamic damage, exacerbating sequelae.
Funding: German Childhood Cancer Foundation, Bonn, Germany.
01 - 03 Oct 2015
European Society for Paediatric Endocrinology